1/42. Absent pituitary gland and hypoplasia of the cerebellar vermis associated with partial ophthalmoplegia and postaxial polydactyly: a variant of orofaciodigital syndrome VI or a new syndrome?We report two sibs with features overlapping those of orofaciodigital syndrome type VI (Varadi syndrome). Both presented at birth with oculomotor abnormalities, dysmorphic facial features, and dysgenesis of the cerebellar vermis. There were minimal oral manifestations (high arched palate) in both of them and one had postaxial polydactyly of both hands and one foot. In addition, there was evidence of aplasia of the pituitary gland on MRI scan in both of them with evidence of hypopituitarism. Both responded well to hormone replacement therapy with improvement in their linear growth and mental ability. These cases may represent a new autosomal recessive midline defect syndrome with features overlapping OFDS VI. Alternatively the features in these children could represent variability within OFDS VI.- - - - - - - - - - ranking = 1keywords = near (Clic here for more details about this article) |
2/42. Application of a rigid endoscope to the microsurgical management of 54 cerebral aneurysms: results in 48 patients.OBJECT: To enhance visual confirmation of regional anatomy, endoscopy was introduced during microsurgery for cerebral aneurysms. The risks and benefits are analyzed in the present study. methods: The endoscopic technique was used during microsurgery for 54 aneurysms in 48 patients. Forty-three aneurysms were located in the anterior circulation and 11 were in the posterior circulation. Thirty-eight aneurysms (70.4%) had not ruptured. All ruptured aneurysms in the present series produced Hunt and Hess Grade I or II subarachnoid hemorrhage. After initial exposure achieved with the aid of a microscope, the rigid endoscope was introduced to confirm the regional anatomy, including the aneurysm neck and adjacent structures. The necks of 43 aneurysms were clipped using microscopic control or simultaneous microscopic/endoscopic control. After clipping, the positions of the clip and nearby structures were inspected using the endoscope. Use of the neuroendoscope provided useful information that further clarified the regional anatomy in 44 cases (81.5%) either before or after neck clipping. In nine cases (16.7%), these details were available only with the use of the endoscope. In five cases (9.3%), the surgeons reapplied the clip on the basis of endoscopic information obtained after the initial clipping. There were two cases in which surgical complications were possibly related to the endoscopic procedures (one patient with asymptomatic cerebral contusion and another with transient oculomotor palsy). CONCLUSIONS: It is the authors' impression that the use of the endoscope in the microsurgical management of cerebral aneurysms enhanced the safety and reliability of the surgery. However, there is a prerequisite for the surgeon to be familiar with this instrumentation and fully prepared for the risks and inconveniences of endoscopic procedures.- - - - - - - - - - ranking = 1keywords = near (Clic here for more details about this article) |
3/42. One and one-half syndrome with supranuclear facial weakness: magnetic resonance imaging localization.OBJECTIVE: To provide clinicoanatomical correlation for a small pontine tegmental ischemic stroke producing the one and one-half syndrome associated with supranuclear facial weakness. DESIGN: Case report. SETTING: Tertiary care center. PATIENT: A 70-year-old man developed left-sided facial weakness sparing the forehead, a left internuclear ophthalmoplegia, and a complete left horizontal gaze palsy immediately after percutaneous transluminal coronary angioplasty. magnetic resonance imaging demonstrated a small lesion in the left paramedian aspect of the dorsal pontine tegmentum. MAIN OUTCOME AND RESULTS: Electromyographic findings were consistent with supranuclear facial involvement. The patient had nearly complete recovery after 1 year. CONCLUSIONS: To our knowledge, this is the first report of supranuclear facial weakness in association with the one and one-half syndrome. The location of the lesion provides evidence of the existence of corticofugal fibers that extend to the facial nucleus in the dorsal paramedian pontine tegmentum.- - - - - - - - - - ranking = 1keywords = near (Clic here for more details about this article) |
4/42. cocaine abuse, generalized myasthenia, complete external ophthalmoplegia, and pseudotonic pupil.We present the case of a 29-year-old woman with generalized myasthenia. Myasthenia with complete external ophthalmoplegia was unmasked by cocaine abuse. It was associated with changes of the pupillary motility, including light-near dissociation and positive 0.1% pilocarpine test. Treatment with acetylcholinesterase inhibitors improved the patient's condition rapidly, and led to complete normalization of extraocular movements and pupillary function. To our knowledge, this is the fourth case of cocaine-related myasthenia, and the first case of myasthenia with pseudotonic pupil.- - - - - - - - - - ranking = 1keywords = near (Clic here for more details about this article) |
5/42. ophthalmoplegic migraine with reversible enhancement of intraparenchymal abducens nerve on MRI.We describe a patient with ophthalmoplegic migraine and right abducens nerve palsy, in whom serial magnetic resonance imaging showed a transient, gadolinium-enhancing lesion in the right lower pons, during both headache and the headache-free period. The enhancing linear lesion was felt to represent intraparenchymal fibers of the affected abducens nerve. The possible pathophysiology of this unique finding is discussed.- - - - - - - - - - ranking = 1keywords = near (Clic here for more details about this article) |
6/42. Upward gaze paralysis as the initial manifestation of hiv-infected patient: a case report.Upward gaze palsy developed in a 23-year-old man without any other abnormal findings except pupillary light-near dissociation in ocular examination. The neuroradiologic examination was consistent with cerebral toxoplasmosis--the most frequent opportunistic infection of the brain in AIDS patients. The serologic work-up revealed a positive hiv test. Symptoms and neuroradiologic abnormality improved after treatment for cerebral toxoplasmosis. hiv infection should be considered in patients who have upward gaze paralysis and neuroimaging compatible with cerebral toxoplasmosis. Empirical treatment may alleviate the upward gaze palsy.- - - - - - - - - - ranking = 1keywords = near (Clic here for more details about this article) |
7/42. Acute bilateral ophthalmoplegia secondary to cavernous sinus metastasis: a case report.A case of a 60-year-old man with metastatic adenocarcinoma of the colon who developed bilateral ophthalmoplegia acutely is presented. Bilateral cavernous sinus metastasis was suggested by magnetic resonance imaging and proven by endoscopic sphenoidal biopsy. The patient was treated with radiotherapy (2000 cGy) with near resolution of his symptoms. This patient represents the first reported case of acute bilateral ophthalmoplegia resulting from hematogenous metastasis to both cavernous sinuses, diagnosed antemortem. A review of the more common causes of acute bilateral ophthalmoplegia is presented.- - - - - - - - - - ranking = 1keywords = near (Clic here for more details about this article) |
8/42. Bilateral medial medullary infarction with oculomotor disorders.BACKGROUND AND PURPOSE: We describe the first case of bilateral medial medullary infarction demonstrated by magnetic resonance imaging. We discuss the relation between this lesion and the oculomotor signs that were observed clinically. CASE DESCRIPTION: A 71-year-old man initially presented with pure motor hemiparesis, which progressed to complete quadriplegia. He also developed nearly complete vertical and horizontal ophthalmoplegia. magnetic resonance imaging revealed upper medial medullary infarctions bilaterally that extended to the pontomedullary junction. CONCLUSIONS: We propose that the vertical oculomotor disorders resulted from involvement of the oculomotor system in the caudal brain stem, especially the caudal paramedian pontine reticular formations on both sides.- - - - - - - - - - ranking = 1keywords = near (Clic here for more details about this article) |
9/42. ophthalmoplegic migraine.ophthalmoplegic migraine is a rare presentation of migraine complicated by an isolated oculomotor paresis. vasodilation of extracranial vessels is believed to underlie the headache, and vasoconstriction to account for the ophthalmoplegia. Whether the vascular insult involves the central or peripheral portions of the oculomotor nerve is still uncertain. We describe a child who presented with ophthalmoplegic migraine and was demonstrated to have a deficiency of the near triad documented by eye movement and pupillary recordings. Voluntary conjugate eye movements--saccades, smooth pursuit, and optokinetic nystagmus--were normal. Vergence amplitudes appropriate to fixation distance were elicited for Snellen optotypes but not to a point source of light. Concurrent measures of pupillary diameter failed to reveal significant modulation to either stimulus. brain magnetic resonance imaging scan was normal, and there was no contrast enhancement of the oculomotor nerve at its exit from the midbrain. Both the oculomotor paresis and concurrent presence of a deficiency of the near triad localized the vascular insult to the oculomotor nerve complex in the brainstem.- - - - - - - - - - ranking = 2keywords = near (Clic here for more details about this article) |
10/42. Fisher's syndrome without total ophthalmoplegia.A 16-year-old boy with acute ophthalmoplegic polyneuritis (Fisher's syndrome) exhibited bilateral internal ophthalmoplegia, but only lateral rectus and superior oblique eye movements were affected. Gaze-evoked nystagmus was also present on left lateral gaze. Pupillary reflex recovered 7 weeks after onset, but neither cholinergic supersensitivity nor light-near dissociation were present during the recovery process. This is the first such case to be reported in which the site of the lesion responsible for the pupillomotor impairment was the Edinger-Westphal nucleus.- - - - - - - - - - ranking = 1keywords = near (Clic here for more details about this article) |
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